Adductor laryngeal dystonia (spastic dysphonia): treatment with local injections of botulinum toxin (Botox)

Adductor spastic dysphonia (SD) is a laryngeal dystonia characterized by a choked, constrained voice pattern with breaks in vocal flow. Treatment with a variety of therapies including speech and pharmacotherapy have minimal benefit; only one-third of patients undergoing recurrent laryngeal nerve section have benefitted at 3 years. We have used local injections of botulinum toxin (Botox) bilaterally into vocalis muscles in 42 patients with SD. Injections were through a teflon-coated hollow electromyography (EMG) recording needle. Unilateral small doses (2.5-3.75 U) were of no clinical benefit. Bilateral small doses resulted in sustained improvement lasting 84.4 +/- 9.3 days. The degree of improvement was 61.1 +/- 4.6%. Common side-effects included a brief period of breathy hypophonia (8.5 +/- 2.5 days) and a mild sensation of choking/aspiration of fluids (1.7 +/- 0.6 days); there were no serious adverse effects. Vocal cord paralysis was not necessary for benefit. Follow-up vocalis muscle EMGs revealed denervation. All patients responded to retreatment (longest follow-up 3.5 years). Patients with prior recurrent laryngeal nerve surgery and residual uncomplicated dysphonia had similar results. Our results indicate that local injection of low-dose Botox is the treatment of choice for SD.     

Orthotopic liver transplantation in a patient with carbamyl phosphate synthetase deficiency and cystic fibrosis

A 15-year-old female with carbamyl phosphate synthetase deficiency, cystic fibrosis, and cystic fibrosis-related diabetes underwent orthotopic cadaveric liver transplantation. Metabolic control was maintained during the procedure with nutritional support and the use of intravenous sodium phenylacetate and benzoate. Her postoperative course was complicated by seizures and a transient decline in her pulmonary function tests, which returned to preoperative levels within one year of the transplant. Now, four years post-transplant, her quality of life has dramatically improved. There are only four Canadian centres with paediatric liver transplantation programs. However, expert medical care for adults with inborn error of metabolism is even more limited, suggesting that access to adult medical care is one of the many factors to be considered when liver transplantation is contemplated for patients with metabolically unstable conditions.     

The effects of token reinforcement on the wardrobe care behavior of emotionally disturbed children

The effectiveness of token reinforcement for improving appropriate wardrobe care in five emotionally disturbed children at a residential treatment center was investigated. In a multiple baseline across subjects design, subjects received token reinforcement for completing six component wardrobe care behaviors. The results indicated that all five subjects increased their appropriate wardrobe behavior during treatment. All but one of the subjects maintained an increase in appropriate wardrobe care behavior during the follow-up.     

Digital papillary adenocarcinoma: a case report.

Digital papillary adenocarcinoma (DPAc) is a relatively rare neoplasm arising from the sweat glands with a predilection for the hand. A case of DPAc in the third finger at the level of the proximal phalanx in a 55-year-old male is presented. Our paper recommends specific consideration of DPAc in evaluating digital soft tissue masses, particularly those that present with an aggressive nature.     

Rationing hospital services in Hong Kong: priority setting by clinicians using the Delphi method.

This article presents the results of a demonstration project of priority setting by clinicians in Hong Kong's public hospitals. Thirty-five chiefs-of-service of public hospitals in Hong Kong participated in a three-round Delphi exercise on identifying interventions that should be charged in public hospitals on the basis of questionable effectiveness and other reasons. A total of 246 interventions were identified in the first round, 126 of which received a positive score (indicating a high degree of consensus amongst the participants) at the end of the final round. The interventions that received higher scores tend to be preventive services, treatment of an elective nature, and procedures for sex-related conditions. As the number of interventions with positive scores in the final round are relatively small, the amount of money to be recovered from charging these interventions is not likely to be substantial. Such results suggest that rationing by itself cannot be a solution to the problems of healthcare financing in Hong Kong.     

Inhibition of rat mammary carcinogenesis by monoterpenoids

We have previously demonstrated the cancer chemopreventive activities of the monocyclic unsaturated monoterpene d-limonene. In the present work we report data evaluating the chemopreventive effects of limonene and five other monoterpenes with various chemical structures using a 7,12-dimethylbenz[a]anthracene (DMBA)-induced rat mammary carcinogenesis model. The terpenes tested include: oxygenated [(-)-menthol] and non-oxygenated (d-limonene) monocyclic forms, oxygenated (1,8-cineole) and non-oxygenated [(+/-)-alpha-pinene] bicyclic forms and oxygenated [(+/-)-linalool] and non-oxygenated (beta-myrcene) acyclic forms. Dietary additions of each of the monocyclic terpenes, d-limonene or (-)-menthol resulted in a significant inhibition of mammary carcinogenesis. Furthermore, menthol was found to be a more potent chemopreventive agent than limonene during the DMBA initiation of rat mammary tumors. The acyclic and bicyclic terpenes had no significant chemopreventive activities at the dose levels used in these studies.